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Kawasaki Disease and Inborn Errors of Immunity: Exploring the Link and Implications

The exact etiopathogenesis of Kawasaki disease (KD), the most common childhood vasculitis, remains unknown; however, an aberrant immune response, possibly triggered by an infectious or environmental agent in genetically predisposed children, is believed to be the underlying pathogenetic mechanism. P...

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Autores principales: Sharma, Saniya, Nadig, Pallavi L, Pilania, Rakesh Kumar, Sharma, Kaushal, Dhaliwal, Manpreet, Rawat, Amit, Singh, Surjit
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10341281/
https://www.ncbi.nlm.nih.gov/pubmed/37443545
http://dx.doi.org/10.3390/diagnostics13132151
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author Sharma, Saniya
Nadig, Pallavi L
Pilania, Rakesh Kumar
Sharma, Kaushal
Dhaliwal, Manpreet
Rawat, Amit
Singh, Surjit
author_facet Sharma, Saniya
Nadig, Pallavi L
Pilania, Rakesh Kumar
Sharma, Kaushal
Dhaliwal, Manpreet
Rawat, Amit
Singh, Surjit
author_sort Sharma, Saniya
collection PubMed
description The exact etiopathogenesis of Kawasaki disease (KD), the most common childhood vasculitis, remains unknown; however, an aberrant immune response, possibly triggered by an infectious or environmental agent in genetically predisposed children, is believed to be the underlying pathogenetic mechanism. Patients with inborn errors of immunity (IEI) are predisposed to infections that trigger immune dysregulation due to an imbalance in various arms of the immune system. KD may develop as a complication in both primary and secondary immunodeficiencies. KD may occur either at disease presentation or have a later onset in IEIs. These include X-linked agammaglobulinemia (XLA), selective IgA deficiency, transient hypogammaglobulinemia of infancy; Wiskott–Aldrich syndrome (WAS), hyper IgE syndrome (HIES); chronic granulomatous disease (CGD), innate and intrinsic immunity defects, and autoinflammatory diseases, including PFAPA. Hitherto, the association between KD and IEI is confined to specific case reports and case series and, thus, requires extensive research for a comprehensive understanding of the underlying pathophysiological mechanisms. IEIs may serve as excellent disease models that would open new insights into the disease pathogenesis of children affected with KD. The current review highlights this critical association between KD and IEI supported by published literature.
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spelling pubmed-103412812023-07-14 Kawasaki Disease and Inborn Errors of Immunity: Exploring the Link and Implications Sharma, Saniya Nadig, Pallavi L Pilania, Rakesh Kumar Sharma, Kaushal Dhaliwal, Manpreet Rawat, Amit Singh, Surjit Diagnostics (Basel) Review The exact etiopathogenesis of Kawasaki disease (KD), the most common childhood vasculitis, remains unknown; however, an aberrant immune response, possibly triggered by an infectious or environmental agent in genetically predisposed children, is believed to be the underlying pathogenetic mechanism. Patients with inborn errors of immunity (IEI) are predisposed to infections that trigger immune dysregulation due to an imbalance in various arms of the immune system. KD may develop as a complication in both primary and secondary immunodeficiencies. KD may occur either at disease presentation or have a later onset in IEIs. These include X-linked agammaglobulinemia (XLA), selective IgA deficiency, transient hypogammaglobulinemia of infancy; Wiskott–Aldrich syndrome (WAS), hyper IgE syndrome (HIES); chronic granulomatous disease (CGD), innate and intrinsic immunity defects, and autoinflammatory diseases, including PFAPA. Hitherto, the association between KD and IEI is confined to specific case reports and case series and, thus, requires extensive research for a comprehensive understanding of the underlying pathophysiological mechanisms. IEIs may serve as excellent disease models that would open new insights into the disease pathogenesis of children affected with KD. The current review highlights this critical association between KD and IEI supported by published literature. MDPI 2023-06-23 /pmc/articles/PMC10341281/ /pubmed/37443545 http://dx.doi.org/10.3390/diagnostics13132151 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Sharma, Saniya
Nadig, Pallavi L
Pilania, Rakesh Kumar
Sharma, Kaushal
Dhaliwal, Manpreet
Rawat, Amit
Singh, Surjit
Kawasaki Disease and Inborn Errors of Immunity: Exploring the Link and Implications
title Kawasaki Disease and Inborn Errors of Immunity: Exploring the Link and Implications
title_full Kawasaki Disease and Inborn Errors of Immunity: Exploring the Link and Implications
title_fullStr Kawasaki Disease and Inborn Errors of Immunity: Exploring the Link and Implications
title_full_unstemmed Kawasaki Disease and Inborn Errors of Immunity: Exploring the Link and Implications
title_short Kawasaki Disease and Inborn Errors of Immunity: Exploring the Link and Implications
title_sort kawasaki disease and inborn errors of immunity: exploring the link and implications
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10341281/
https://www.ncbi.nlm.nih.gov/pubmed/37443545
http://dx.doi.org/10.3390/diagnostics13132151
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